Incidence Of Idiopathic Inflammatory Myopathies Research Articles

The spectrum of idiopathic inflammatory myopathies in Western Australia: epidemiological characteristics and mortality over time

To determine long term overall and subgroup specific incidence rates and associated mortality for idiopathic inflammatory myopathies (IIM) in a population wide study. We included patients hospitalised between 1980 and 2015 with incident IIM as defined by relevant diagnostic codes for dermatomyositis (DM) polymyositis (PM), inclusion body myositis (IBM), other IIM and overlap myositis (OM) in the Western Australia Health Hospital Morbidity Data Collection (n = 846). Trends over time for annual incidence rate per million population (AIR) were analysed by least square regression and Kaplan–Meier survival and mortality rates (MR)/100 person years compared with a matched control group (n = 3681). The averaged AIR for all IIM was 19 (CI 10.4–27.5) and stable over time with point prevalence reaching 205.3 (CI 185.6–226.6) per million in 2015. Over time, the AIR for DM 5.0 (CI 0.6–9.4) and IBM 3.3 (CI 0.7–9.6) was stable, while AIR decreased for PM (p < 0.01) and increased for other IIM (p < 0.01) and OM (p < 0.01). IBM patients were eldest at diagnosis (68 years, CI 59–77) with male preponderance in IBM (53.4%) and other IIM (55.8%) groups. Crude mortality (54.5 vs 41.3%), MR ratio (6.65 vs 5.91) and 5 (65.8% vs 71.6%) and 10-year (52.5% vs 58.7%) survival were all worse for IIM patients (all p < 0.05). IBM patients had highest MR (10.1; CI 8.38–12.14) and lowest 10-year survival (39.2%). While cardiovascular disease and cancer were predominant causes of death, they were proportionally lower in IIM patients, where respiratory and rheumatic disease were more frequent causes of death. While the overall incidence of IIM in WA was stable over 35 years, the spectrum of IIM has changed significantly with increases especially in other IIM and OM. The overall prognosis with IIM remains guarded with 10-year survival just over 50%.

Epidemiology of the idiopathic inflammatory myopathies.

The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of systemic autoimmune diseases that affect the skeletal muscles and can also involve the skin, joints, lungs and heart. The epidemiology of IIM is obscured by changing classification criteria and the inherent shortcomings of case identification using healthcare record diagnostic coding. The incidence of IIM is estimated to range from 0.2 to 2 per 100,000 person-years, with prevalence from 2 to 25 per 100,000 people. Although the effects of age and gender on incidence are known, there is only sparse understanding of ethnic differences, particularly in indigenous populations. The incidence of IIM has reportedly increased in the twenty-first century, but whether this is a genuineincrease is not yet known. Understanding of the genetic risk factors for different IIM subtypes has advanced considerably. Infections, medications, malignancy and geography are also commonly identified risk factors. Potentially, the COVID-19 pandemic has altered IIM incidence, although evidence of this occurrence is limited to case reports and small case series. Consideration of the current understanding of the epidemiology of IIM can highlight important areas of interest for future research into these rare diseases.

The incidence of idiopathic inflammatory myopathies in the adult Slovenian population

Idiopathic Inflammatory myopathies (IIM) are rare disorders. The aim of our study was to determine the incidence of IIM in a well-defined Slovenian region. This retrospective study was conducted at the Department of Rheumatology, University Medical Centre Ljubljana, the only secondary/tertiary rheumatology center in a region with a population of 704,342 adults. We identified potential IIM cases by searching the electronic patient records for ICD-10 codes M33, M35.1, M35.8, M60, G72, G73, and J84. We included incipient IIM cases between January 2010 and December 2017, who were at the time of the diagnosis, residents of the inspected region. To avoid under-reporting due to miscoded cases, we obtained a list of the patients who had histological patterns consistent with IIM on muscle biopsy from the Institute of Pathology. The annual incidence rate for IIM was calculated. During the eight-year observation period, we identified 65 IIM cases (72.3% female, median (IQR) patient age 64.8 (54.8-73.2) years). The estimated annual incidence of IIM in the studied population was 11.5 (95% CI 9.0-14.6) per 106 adults, in females 16.2 (95% CI 12.1-21.4), and in males 6.6 (95% CI 4.0-10.2) per 106 adults. The incidence rate of IIM in Slovenia is consistent with data from the literature.

FRI0447 Epidemiology of idiopathic inflammatory myopathies in england – a database analysis

BackgroundThe idiopathic inflammatory myopathies (IIM) are a rare, heterogeneous group of autoimmune muscle disorders, including dermatomyositis (DM), polymyositis (PM), and inclusion body myositis. Data on the epidemiology of IIM are...